July 28, 1998

ESSAY

When Medicine Goes Too Far in the Pursuit of Normality

By ALICE DREGER

realized recently that I suffer from a genetic condition. Although I have not actually had my genome screened, all the anatomical signs of Double-X Syndrome are there.
And while I could probably handle the myriad physiological disorders associated with my condition -- bouts of pain and bleeding coming and going for decades, hair growth patterns that obviously differ from "normal" people's
-- the social downsides associated with it are troubling.

One person's abnormality is another person's life.

Even since the passage of the Americans With Disabilities Act, people with Double-X remain more likely than others to live below the poverty line, more likely to be sexually assaulted, and are legally prohibited from marrying people with
the same condition. Some potential parents have even screened fetuses and aborted those with Double-X in an effort to avert the tragic life the syndrome brings. Perhaps you know Double-X by its more common name: womanhood.

This fact of my "genetic condition" came to me one evening as I sat in a conference room of our local hospital participating in a community dialogue sponsored by the Human Genome Project. Our group had just finished reading a rather
bleak description of the anatomy and life of the "average" woman with Turner's syndrome: "webbed neck, short stature, no chance for bearing children" and so on. Turner's syndrome, which affects roughly one
in 2,500 girls born each year, arises when a person is born with a single X chromosome and no Y. It is, essentially, a "single-X syndrome."

Our group was discussing the genetic screening of pre-implantation embryos, and given the depressing description of Turner's provided to us, no wonder most everyone in the room, pro-choicers and pro-lifers alike, saw Turner's as
a sad genetic disease.

But I had by then been studying human intersexuality -- anatomical sexual variations, including Turner's -- for several years, and had talked with and read the biographies and autobiographies of many people born intersexed. I knew that
women with Turner's would describe their lives with more balance. So I began to think about how a woman with single-X and a sense of humor could describe the life of those of us women with double-X, and came up with the above portrayal.

My point is not that people with unusual genetic conditions do not suffer more than those without them; clearly, many do. But I am troubled that I see ever more cases in which psychosocial problems caused by stereotypes about anatomy are being
"fixed" by "normalizing" the anatomy. There are serious downsides to this, both for the person being "normalized" and for those around her.

Take for example the "treatment" of short stature. Some pharmaceutical companies and physicians have advocated giving human growth hormone injections to very short children in an attempt to help them grow taller than they otherwise
would. Profound short (and tall) stature may lead to disorders like back problems because our culture structures the physical world for average-height adults. But no one advocates using the hormone to prevent back problems.

Hormone treatments are used because of the presumption that an adult of short stature will not fare as well socially as one of average size. Indeed, statistically, taller men are more likely than shorter men to be hired, given a raise or elected
President.

Average-sized people see short people and tend to feel sorry for them. Getting short children to grow more seems pretty beneficent.

The same kind of intended beneficence drives the medical management of children born intersexed. Many physicians assume that intersexed children, with their unusual genitalia, will be rejected by family and peers. So they recommend early cosmetic
surgery to try to erase the signs.

What's wrong with these "normalization" technologies? First, it isn't clear that they work. It seems that if an unusual anatomy leads to a psychosocial problem, "normalizing" the anatomy should solve the problem.
But of the few follow-up studies that have been done on intersex surgeries, none examine the psychological well-being of the subjects in any real depth. Most simply report on the status of the postsurgical anatomy, while a very few report
on whether the subjects are married (psychological health is presumed from this).

Psychological follow-up on the growth hormone treatments is similarly lacking. A study published in the March 28 Lancet reported that a randomized trial of the hormone in "short normal girls" resulted in the treated girls' averaging
a final height of almost three inches more than the control group, but added that "no significant psychosocial benefits have yet been shown."

Perhaps we should not expect measurable psychological benefits from a gain of about three inches. Yet recall that the entire "normalization" treatment for short stature was designed to produce psychological benefits.

More worrisome than the loss of focus in these studies is the fact that these treatments often backfire. Children subjected to these kinds of treatments often report feelings of inadequacy and freakishness as a direct result of their parents'
and doctors' attempts at normalization. And the treatments are not without physical risks. For example, intersex surgeries all too frequently leave scarred, insensate, painful and infection-prone genitalia.

So anatomy-focused have we become that children with unusual conditions are often not provided any professional psychological counseling.

Nor are their parents, who are dealing with their own feelings of confusion, shame, grief and worry. The result is the message that the problem is primarily anatomical and, by consequence, a "fault" of the child and perhaps also
the parents.

By extension, a dictate then kicks in: If you can fix it, you should. A friend of mine recalls the time a plastic surgeon came up to him at a party, looked at his nose and said, "You know, I can fix that." When my friend said, "No
thanks," the plastic surgeon appeared to think my friend a little crazy. As normalizing technologies become more accessible, people are expected to be bothered by their "unusual" features and expected to want to fix them.

Some surgeons say they normalize intersexed children because it is too hard to be different. One points out that we still live in a nation where dark-skinned people have a harder time than light-skinned people do. But would he suggest we work
on technologies to "fix" dark skin? Would we call people who refuse to lighten their children cruel Luddites?

The funny thing is, when I ask people with dark skin if they would change their color, they tell me no, and when I ask women if they would rather be men, they tell me no, and I get the same response when I ask people with unusual anatomies
if they would take a magic pill to erase their unusual features.

They tell me, instead, that they would support an end to social stereotypes and oppression, but that they would not trade themselves in for a "better" model. This sentiment even comes from conjoined twins. Chang and Eng Bunker, the
conjoined brothers born early in the 19th century and dubbed the Siamese twins, confessed that they preferred their state because it enabled them to bring a "double strength and a double will" to each purpose. Similarly, women
born with big clitorises confess to liking their unusual anatomy. But this is the absolutely forbidden narrative -- not only rejecting normalization but actively preferring the "abnormal."

What do I suggest? First, a few basic realizations. In spite of medical advances, unusual anatomies generally cannot be fixed in any significant way without significant risk, and that risk, when medically unnecessary, should be approved by
the person at risk.

We also need to remember that just because it makes sense that you ought to be able to fix anatomically based psychosocial problems anatomically, that doesn't mean it is so. Working to eliminate social stereotypes would be more effective
and better for everyone in the long run. I do not wish to see options entirely withdrawn from mature patients; I am suggesting we slow down the normalization of children, many of whom are likely to gain much more from acceptance and psychological
support than injections and scalpels.

But how do we fix the social problems? When I talk about intersex, people ask me, "But what about the locker room?" Yes, what about the locker room? If so many people feel trepidation around it, why don't we fix the locker room?
There are ways to signal to children that they are not the problem, and normalization technologies are not the way.

Instead of constantly enhancing the norm -- forever upping the ante of the "normal" with new technologies -- we should work on enhancing the concept of normal by broadening appreciation of anatomical variation. Show potential parents,
medical students and genetic counselors images of unusual anatomies other than the deeply pathologized ones they are typically given. Allow those with the unusual anatomies to describe their own lives in full and rich detail. Even let
them tell the forbidden narrative of enjoying their Double-X Syndromes.

Alice Dreger, professor of science and technology at Michigan State University, wrote ``Hermaphrodites and the Medical Invention of Sex,'' (Harvard University Press, 1998).

ESSAY is published weekly, on Tuesdays. Click here for a list of links to other columns in the series.